Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study.
Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study.
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卵巢性索肿瘤伴环状小管的治疗和预后：一项回顾性研究
DOI：
10.1186/s12885-015-1277-y
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发表时间：
2015-04-12
期刊：
影响因子：
3.8
通讯作者：
Shen K
中科院分区：
文献类型：
--
作者：
Qian Q;You Y;Yang J;Cao D;Zhu Z;Wu M;Chen J;Lang J;Shen K
背景由于性索环状小管性肿瘤(SCTAT)的罕见，临床上难以识别，目前尚无规范的治疗方法。本研究旨在探讨卵巢SCTAT患者的治疗效果和预后。方法对北京协和医院收治的13例卵巢SCTAT患者进行队列研究。结果SCTAT占卵巢性索间质瘤的1.4%，平均发病年龄22.6岁。所有患者在发病时均出现月经紊乱或同性性早熟。11例行单侧输卵管卵巢切除术。复发率为46.2%，38.5%的患者多次复发。随着复发次数的增加，无病间隔逐渐缩短。复发肿瘤多与原发灶同侧，位于腹膜后。手术仍是复发病例的主要治疗方法。血清雌二醇和孕酮水平通常在发病时升高，术后急剧下降，随着复发的发展再次升高。中位无进展生存期(PFS)为97.8个月，1年和5年PFS分别为92%和67%。5年总生存率为100%。结论单侧输卵管卵巢切除术是治疗包膜完整、无PJS的原发SCTAT的一种可行方法。对于复发病例，建议彻底切除肿瘤，并进行长期随访。尽管复发风险很高，但SCTAT的预后相对较好。
BackgroundOwing to the rarity of sex cord tumor with annular tubules (SCTAT), it is difficult to recognize SCTAT clinically and there is no standard treatment. The aim of our study was to investigate the treatment outcomes and prognosis of patients with ovarian SCTAT.MethodsA cohort of 13 patients with SCTAT diagnosed and treated in Peking Union Medical College Hospital was studied. Data on clinicopathological characteristics, treatment, and prognosis were retrospectively reviewed and analyzed.ResultsSCTAT accounted for 1.4% of ovarian sex cord stromal tumors, with an average onset age of 22.6 years. All patients presented with menstrual disturbances or isosexual precocity at disease onset. Initial surgery was unilateral salpingo-oophorectomy in 11 cases. Recurrence rate was 46.2%, and 38.5% of patients experienced multiple recurrences. The disease free interval gradually shortened with increasing numbers of recurrences. Recurrent tumors were mostly ipsilateral to the primary tumor and located in retroperitoneum. Surgery remained the main treatment for recurrent cases. Serum estradiol and progesterone levels usually elevated at disease onset, decreased dramatically after operation, and they elevated again with the development of recurrence. The median progression-free survival (PFS) was 97.8 months, and the 1-year and 5-year PFS were 92% and 67%, respectively. Five-year overall survival (OS) was 100%.ConclusionsUnilateral salpingo-oophorectomy is a feasible treatment for primary SCTAT cases with intact capsules and without PJS. Complete tumor resection is suggested for recurrent cases and long-term follow-up is strongly recommended. Despite the high risk of recurrence, SCTAT prognosis is relatively favorable.
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影响因子：
3.4
作者：
DOLAN, J;ALTIMIMI, AH;HASLETON, PS
通讯作者：
HASLETON, PS
影响因子：
7.5
作者：
Lele, SM;Sawh, RN;Krigman, HR
通讯作者：
Krigman, HR
DOI：
10.4103/2229-516x.117093
发表时间：
2013-07
影响因子：
1.1
作者：
Momin YA;Kulkarni MP;Pandav AB;Sulhyan KR
通讯作者：
Sulhyan KR
影响因子：
4.7
作者：
Brown, Jubilee;Sood, Anil K.;Gershenson, David M.
通讯作者：
Gershenson, David M.
影响因子：
1.8
作者：
Bercaw, Jennifer L;Sanchez, Judith;Dietrich, Jennifer E
通讯作者：
Dietrich, Jennifer E